ORTOPEDİK CERRAHİDE LOWE SENDROMU TANILI HASTADA ANESTEZİ YÖNETİMİ

Lowe sendromu X'e bağlı resesif kalıtılan, seyrek görülen, ciddi göz, sinir sistemi ve böbrek patolojilerinin eşlik ettiği bir hastalıktır. Doğumda var olan patolojilerin yanında yıllar geçtikçe tabloya eklenen anomaliler de görülür. Biz bu raporda, özellikle konjenital katarakt, kognitif bozukluklar ve renal tubuler asidoz ile karakterize olan Löwe Sendromunda, bilateral genu valgum nedeniyle ortopedik girişim yapılacak hastanın anestezik yaklaşımını literatür ışığında tartışmayı amaçladık. 

ORTOPEDİK CERRAHİDE LOWE SENDROMU TANILI HASTADA ANESTEZİ YÖNETİMİ

Lowe syndrome is a rare, X-linked recessive inherited, disease which is associated with serious eye, nervous system and nephrological pathologies. Co-morbidities add over the years to the pathologies seen at birth. In this report we aimed to discuss the anesthetic implications of Lowe Syndrome, which is characterized by especially congenital cataracts, renal tubular acidosis and cognitive impairment, for the patient who was scheduled for bilateral genu valgum surgery, in the light of the literature.

___

  • Abbassi V, Lowe UC, Calcagno PL. Oculo-cerebro- renal syndrome. Am J Dis Child 1968; 115: 145–168
  • Amirhakimi G., Fallahzadeh MH., Saneifard H. Lowe Syndrome:Report of a case and brief literature review. Iranian Journal of Pediatrics 2009; 19(4):417-420. 3. Badrinath SK, Braverman AD.Alfentanil and sulfentanil prevent the increase in IOP 1988;67:S5. B, Ivankovich from
  • Succinylcholine.Anesth Analg
  • Bockenhauer D,, Bokenkamp A, Van't Hoff W,, Levtchenko E, ,Kist-van Holthe JE, Tasic V, ,Ludwig M. Renal phenotype in Lowe Syndrome: a selective proximal tubular dysfunction.Clinical Journal of the American Society of Nephrology, September 2008, Pages: 1430-1436
  • Gropman A, Levin S, Yao L, Lin T, Suchy S, Sabnis S, Hadley D, Nussbaum R. Unusual renal features of Lowe syndrome in a mildly affected boy. Am J Med Genet 2000; 95(5):461-6
  • Hisako Komatsu, Masatomo Sakakibara, Yutaka Yoshimura et al. Anaesthetic management for a patient Anaesthesia. 1994;8:121–123. syndrome. Journal of
  • Laing CM , Unwin RJ, Renal tubuler asidosis. J Nephrol 2006. Mar-Apr; 19.
  • Laube GF, Russell-Eggitt IM, van’t Hoff WG. Early proximal tubular dysfunction in Lowe syndrome. Arch Dis Child. 2004; 89(5)
  • Loi M. Lowe syndrome. Orphanet J Rare Dis Orphanet J Rare Dis. 2006 May 18;1:16.
  • Richards W, Donnel GN, Wilson WA, Stowens D, Perry T. The oculocerebrorenal syndrome of Lowe. Am J Dis Child. 1965.
  • Saricaoglu F, Demirtas F, Aypar U. Preoperative and perioperative management of a patient with LOWE syndrome diagnosed to have Fanconi’s syndrome. Pediatric Anesthesia. 2004;14:530–532
  • Sessler DI (1990) Temperatur monitoring. In Miller RD Anesthesia, New York, 1227-1240